Counseling Families of Patients with Hypoplastic Left Heart Syndrome

Counseling Families of Patients with Hypoplastic Left Heart Syndrome 150 150 Mary Bates, PhD

A new survey investigates how palliation strategies have changed in the last ten years.

Approximately ten years ago, researchers at Nationwide Children’s Hospital surveyed pediatric cardiac providers regarding initial counseling for families of patients with hypoplastic left heart syndrome (HLHS). Given the evolution of options and outcomes since that time, the team recently queried providers again to examine current practices. The survey results are reported in the journal Pediatric Cardiology.

There are five initial pathways for patients born with HLHS, each having advantages and disadvantages: Norwood palliation with a modified Blalock-Taussig-Thomas shunt (NW-BTT), Norwood palliation with a right ventricle to pulmonary artery conduit (NW-RVPA), the hybrid procedure, heart transplantation, or non-intervention/hospice care.

For the current study, the researchers compared survey responses solicited in 2021 to responses to identical questions from 2011. They received 322 responses to the new survey, mostly from cardiologists but also including cardiothoracic surgeons and nurse practitioners.

Counseling Families of Patients with Hypoplastic Left Heart Syndrome

The results showed that the NW-RVPA procedure has become the most recommended strategy throughout the US for standard risk HLHS patients, though the NW-BTT procedure is still widely performed.

For HLHS patients with comorbid conditions, recommendations tended to be more variable, with broader use of the hybrid procedure, especially in low-birth weight infants. Non-intervention has become a recommendation for those infants with severe end organ dysfunction or chromosomal abnormality. Nonintervention was still offered as a possible option (71.4%) to the majority of standard risk patients.

Nationwide Children’s pioneered the use of the hybrid procedure strategy and is a major proponent of its use in the appropriate population, says Deipanjan Nandi, MD, a cardiologist with the Pediatric & Adult Advanced Cardiac Therapies Program at Nationwide Children’s and lead author of the study. “It is interesting to see that other centers are increasingly offering the hybrid procedure for infants who are premature or have other complications.”

Additionally, the survey showed that about 70% of providers counsel families that patients with HLHS will likely have neurologic and physical deficits compared to peers, and that this percentage has not changed significantly since the previous survey.

Dr. Nandi says that eventual neurologic and physical outcomes may differ between the palliation strategies, but further long-term data are needed.

“Better data collection and characterization of long-term outcomes are going to help us understand the eventual neurologic and physical outcomes for patients undergoing different procedures,” he says.

“Thankfully, we’re in an era of increasing data sharing, which will help us take better care of these patients and provide better counseling for families facing these very difficult decisions.”


Nandi D, Culp S, Yates AR, Hoffman TM, Juraszek AL, Snyder CS, Feltes TF, Cua CL. Initial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome: 2021 vs 2011. Pediatr Cardiol. 2023 Jun;44(5):1118-1124. doi: 10.1007/s00246-023-03170-5. Epub 2023 Apr 26.

About the author

Mary a freelance science writer and blogger based in Boston. Her favorite topics include biology, psychology, neuroscience, ecology, and animal behavior. She has a BA in Biology-Psychology with a minor in English from Skidmore College in Saratoga Springs, NY, and a PhD from Brown University, where she researched bat echolocation and bullfrog chorusing.